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  2. 2. A 16 yrs old female patient was admitted with c/o  Breathlessness since 2 months  Swelling over lower limbs since 15 day  Decreased urine output since 8 days  h/o fever since 1 month +  No h/o cough/chest pain/hemoptysis  No h/o joint pain  No h/o intermittent claudication  No h/o DM/PTB /RHD in past
  3. 3. General examination  Conscious and oriented  febrile  Pulse -104/min in rt radial,regular,bounding lt radial weak ,no r-r delay. femorals and popliteals dorsalis pedis palpable, no radio-femoral delay.  Blood pressure(mmHg) :- RT. UL =210/110 LT. UL =180/100  RT. And LT. LL =240/130  JVP –raised  Edema feet +  pof +  Pallor +  No icterus/cynosis/clubbing
  4. 4. Systemic examination  CVS:- PSM + III/VI AT APEX  RS:-Basal crepitations present  PA :-liver palpable 4cm /tender Abdominal bruit +  CNS :- NAD
  5. 5. Investigations  Hb=8.8 gms%  TLC=7000/cumm P=69%,L=27%,E=2%,M=2%  PS:-RBC‟S were normocytic normochromic with mild anisopoikilocytosis,platelets –adequate,no parasite  ESR =36 mm in 1st hr.  URINE :Protein ++,sugar-nil,5-6 pus cells ,10-12 rbcs  KFT: –BUL=265mg%,Sr.Cr.=3.5mg%  Na =122 ,K=3.8 meq/l  LFT:-TP =6.2, TB=2.7,ALP=325,OT =66,PT=268  ANA :-0.29 OD RATIO
  6. 6. Investigations  ECG:- S/O LVH  X-RAY CHEST PA s/o cardiomegaly  2-D ECHOCARDIOGRAPHY s/o LVH, LV hypokinesia,moderateMR,severe TR ,severe PH, LVEF =20%.  RENAL DOPPLER S/O obscured rt. Renal artey origin  AV DOPPLER :Both upper and lower limb AV Doppler was wnl except low psv biphasic waveform lt. upper limb system.
  7. 7. ECG
  8. 8. X- Ray chest
  10. 10. MR Renal angiography  Study revels Type B standford aortic dissection at level of origin of renal arteries ,2 cm in diameter and 2.1 cm in length  Lt. renal artery arising from true lumen  Rt. Renal artery arising from false lumen with significant reduction in caliber.  Lt. kidney measure 9.6/4.7/5.2 cms and artery measure 6mm at ostia,3mm at hilum.  Rt . Kidney measure 7.2/3.6/3.3cms and artery measure 2.5mm at ostia,1.8mm at hilum
  11. 11. MR Renal angiography
  12. 12. MR Renal angiography
  13. 13. MR Aortography  Narrowing of mid 1/3 descending aorta for length 21 mm and 7 mm diameter.  No e/o collaterals  f/s/o POST DUCTAL COARCTATION OF AORTA.
  14. 14. MR Aortography
  16. 16. Treatment  ANTIHYPERTENSIVE :-Tab Nicardepine 20 mg BD Tab Metoprolol 50 mg OD Tab Prazocin 5 mg BD  DIURETICS :- Inj. Lasix 40 mg BD  CVTS OPINION :-Advised endovascular stenting.
  17. 17. DISCUSSION
  18. 18. Introduction  Takayasu‟s arteritis (TA) is an inflammatory vascular disease of the young involving the large elastic arteries resulting in occlusive or ecstatic changes mainly in the aorta and its major branches as well as the pulmonary artery and its branches.  Also known by “pulseless disease”, “occlusive thromboaortopathy”, and “Martorell syndrome”,  predilection for females with wide geographical variations  M : F ratio in 1 : 6.4 in india  TA may be diagnosed from childhood to late adult life,  Rarely occurs over 40 years of age
  19. 19. History  1827 by R. Adams who was the first physician to note absence of pulse in all four extremities  In1856 Savory reported a case of young female with absence of pulses.  In 1908, Mikito Takayasu, a Japanese opthalmologist described a peculiar wreath-like appearance of retinal blood vessels with absence of radial pulse.
  20. 20. Pathophysiology  All the three layers causing extensive intimal proliferation, inflammation of media and adventitia followed by marked fibrous scarring.  In older cases, hyalinization of deeper layers of intima and dystrophic calcification can be seen.  Skipped areas of aortic involvement are quite characteristic of aortoarteritis.  Histopathologically, adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum, mononuclear inflammation, and rarely granulomatous modifications.  The subclavian artery, common carotid artery, vertebral artery, and renal artery are more often affected.
  21. 21. Clinical presentation  Early phase or pre pulseless phase described by nonspecific systemic features (malaise, arthralgia mild synovitis, weakness, myalgias weight loss, and low-grade fever),  Pulseless phase (vascular inflammatory phase) is characterized by claudication, headaches, dizziness, and amaurosis or diplopia, difficulty in looking upwards, renovascular hypertension, chest pain or palpitation, pulmonary (dyspneea,hemoptysis and pleurisy), gastrointestinal (anorexia, nausea), and skin (rare, ulcers).  Occlusive phase: usually involves common carotid artery (visual defects, strokes, TIA), vertebral artery (dizziness, visual defects), subclavian artery (arm claudication), aorta (aortic regurgitation,cardiac heart failure), pulmonary artery, cardiac and celiac axis, renal artery (renovascular hypertension), iliac artery (claudication).
  22. 22. Diagnosis criteria  The diagnosis of TA is largely based on the combination of 1) clinical manifestations, 2) laboratory evaluation, and 3)diagnostic imaging.  When the diagnosis is presumed, it is typically confirmed by a radiographic technique.
  23. 23. American College OF Rheumatology Classification Criteria to be diagnosed with Takayasu’s arteritis must meet 3 out of 6 criteria:  Age under 40 at disease onset;  Claudication of extremities;  Decreased branchial artery pulse;  Blood pressure difference>10 mmHg between arms;  Bruit over subclavian arteries or aorta;  Arteriogram abnormality: occlusion or narrowing in aorta or main branches.
  24. 24. Diagnostic criteria by Ishikawa (1988).  These consist of one  obligatory criterion (age <40 years at diagnosis or onset of disease),  two major criteria (left and right mid subclavian artery lesions) and  nine minor criteria  (high ESR, common carotid artery tenderness, hypertension, aortic regurgitation or annulo-aortic ecatisa, lesions of pulmonary artery, left mid common carotid artery, distal branchio-cephalic trunk, thoracic aorta and abdominal aorta).  In addition to the  obligatory criterion, the presence of two major criteria , or one major plus two or more minor criteria; or four or more minor criteria suggests a high probability of the presence of aortoarteritis.
  25. 25. The Ishikawa diagnostic criteria modified by Sharma et al 1995  Type I -only the branches of the aortic arch.n  Type IIa - aorta only at its ascendingportion and/or at the aortic arch. The branches ofthe aortic arch may be involved as well. The rest ofthe aorta is not affected.  Type IIb - the descending thoracic aorta withor without involvement of the ascending aorta orthe aortic arch with its branches. The abdominalaorta is not involved.  Type III is concomitent involvement of thedescending thoracic aorta, the abdominal aorta,and/or the renal arteries. The ascending aorta, theaortic arch, and its branches are not involved.  Type IV involves only the abdominal aorta and/orthe renal arteries.  Type V is a generalized type, with combined
  26. 26. Sharma modified criteria for clinical diagnosis of TA Major criteria  1. Left midsubclavian artery lesion: stenosis or occlusion 1 cm proximal to the left vertebral artery orifice up to 3 cm distal  2. Right midsubclavian artery lesion: stenosis or occlusion from the right vertebral artery orifice to 3 cm beyond  3. Characteristic signs and symptoms (> 1-month duration)  A. Limb claudication  B. Pulselessness or blood pressure differential > 10 mmHg in arms  C. Exercise ischaemia  D. Neck pain  E. Fever  F. Amaurosis fugax  G. Syncope  H. Dyspnoea  I. Palpitations  J. Blurred vision
  27. 27. Minor criteria  1. High ESR: Westergren ESR > 20 mm/h  2. Carotodynia  3. Hypertension: brachial blood pressure > 140/90 mmHg o popliteal blood pressure > 160/90 mmHg  4. Aortic regurgitation or annuloaortic ectasia: determined by auscultation, arteriography or echocardiography  5. Pulmonary artery lesion: lobar or segmental artery occlusion, or stenosis or aneurysm of pulmonary trunk  6. Left middle common carotid artery lesion: stenosis or occlusion of middle 5 cm portion starting 2 cm from its orifice  7. Distal innominate artery lesion: stenosis or occlusion in the distal third  8. Descending thoracic aorta lesion: narrowing, aneurysm, or luminal irregularity  9. Abdominal aortic lesion: narrowing, aneurysm, or luminal irregularity  10. Coronary artery lesion: documented by arteriography in patients < 30 years of age and without risk factors for atherosclerosis  Two major, or one major and two minor, or four minor criteria indicate a high probability of Takayasu‟s arteritis.
  28. 28. Clinical Classification 2004  Ueno et 1967 classified it into three types.  Type-I, localized to the arch of aorta and its branches.  Type-II,involve the thoracoabdominal aorta and its branches without involvement of the arch.  Type-III is combined features of both Type-I and Type-II.  Type-IV in which there is pulmonary involvement in addition to features of Type-I, II or III.( Lupi Harrera et al in 1975)  Type- V proposed by Panja et al (2004) and it indicates involvement of coronary arteries.
  29. 29. Incidence The distribution of various types is not uniform globally. As far as India is concerned, Agarwal et al reported among North Indian patients the  Incidence of various types as  Type-I - 22%,  Type-II - 25%,  Type-III - 53% and  Type-IV - 26%. Panja et al involving 650 patients belonging to Eastern India and Bangladesh (largest series from India) revealed  Type- I - 16%;  Type-II - 8%;  Type-III - 76%;  Type-IV - 36% and  Type-V - 10%.
  30. 30. Imaging  Angiography is investigation of choice for the diagnostic evaluation of TA  Echocardiography and Duplex Doppler ultrasonography are the first investigations and the characteristic findings of TA consist of wall thickening,stenosis, aneurysms, calcifications, occlusions, and pulsatility.  Computed tomography (CT) and magnetic resonance imaging (MRI) are the best diagnostic techniques for assessing pulmonary vascular lesions  PET technique or FDG-PET ((18) F-fluorodeoxyglucose positron emission tomography permits an early diagnosis of TA during the inflammatory or „„pre-pulseless‟‟ phase
  31. 31. Treatment  High-dose corticosteroids are the mainstay of TA therapy if caught early ; consists of prednisone 1 mg/kg/day given for the first 1–3 months, then reduction after an alternate-day  Cytotoxic therapy is mainly used in cases with ongoing disease activity, regardless of glucocorticoid therapy.  cyclophosphamide (1-2 mg/kg/day), azathioprine (1-2 mg/kg/day), or methotrexate (0.3 mg/kg/week)  Recently, mycophenolate mofetil, and anti-tumour necrosis factor alpha biological agents have been proposed in patients unresponsive to other immunosuppressor agents
  32. 32. Surgical care Indications for surgical repair or angioplasty are as follows: 1.Renovascular stenosis causing hypertension 2.Coronary artery stenosis leading to myocardial ischemia 3.Extremity claudication induced by routine activity 4.Cerebral ischemia and/or critical stenosis of 3 or more cerebral vessels 5.Aortic regurgitation 6.Thoracic or abdominal aneurysms larger than 5 cm in diameter 7.Severe coarctation of the aorta 8.Percutaneous transluminal coronary angioplasty is followed by restenosis at the angioplasty site within 1-2 years in a substantial number of patients.
  33. 33. References:  TAKAYASU’S ARTERITIS – AN UPDATE- Daniela Bartoº1,Ecaterina Bontaº1,S. Ghiorghe1 Cercetãri Experimentale & Medico-Chirurgicale Anul XIII l Nr3- 4/2006 l Pag. 149-152  Takayasu’s arteritis: a review of the literature -Silvia Maffei, Michela Di Renzo, (Intern Emerg Med 2006; 1 (2): 105-112  Current Status of Aortoarteritis in India Manotosh Panja*, PC Mondal**  Aortoarteritis presenting as coarctation of aorta.- Bahuleyan CG, Bhuvaneswaran KP
  34. 34. Thanks !